medigraphic.com

2011, Number 2

<< Back Next >>

Cir Cir 2011; 79 (2)

Duodeno-biliary obstruction in Peutz-Jeghers syndrome

Pérez-Torres E, Fosado-Gayosso M, Gil-Rojas N, Higuera-de la Tijera MF

Language: Spanish
References: 21
Page: 186-190
PDF size: 359.40 Kb.


ABSTRACT

Background: Peutz-Jeghers syndrome (PJS) is a pathology with autosomal dominant inheritance characterized by the presence of hamartomatous polyposis and mucocutaneous pigmentation. We present a case report from the Hospital General of Mexico.
Clinical case: We present the case of a 28-year-old male. During physical examination we noted hyperpigmented dermatosis of the oral mucosa and lips. The same condition was seen in both palms. The condition evolved with intolerance to oral feeding and progressive obstructive jaundice. Panendoscopy reported pangastric sessile polyps, as well as being pylorus passable. In the second duodenal portion occupying the region of the ampoule of Vater was a sessile polyp that deformed the region. Exit of bile was not observed through the ampoule. Ultrasound and computed tomography of the abdomen corroborated dilatation of the extrahepatic biliary tract. Two endoprostheses were placed in the bile duct by endoscopic cholangiography, with improvement of biliary obstruction. Roux-en-Y gastrojejunoanastomosis was performed because of obstruction of the duodenum by polyps between the second and third portion. Jejunal enterotomy was necessary because of the presence of intraluminal injury formed by a conglomerate of polyps. The patient had a satisfactory evolution. Pathological study reported hamartomatous polyps.
Conclusions: Duodenal obstruction secondary to biliary tract obstruction is a rare manifestation associated with PJS. In these cases, the treatment of choice is polyp resection using endoscopic and/or surgical approach as well as management of the biliary tract obstruction.


REFERENCES

  1. McGarrity TJ, Kulin HE,Zaino RJ.Peutz-Jeghers syndrome. Am J Gastroenterol 2000;95:596-604.

  2. Choi HS. Peutz-Jeghers syndrome: a new understanding. J Korean Med Sci 1999;14:2-7.

  3. Lundell LR, Dent J, Bennett JR, Blum AL, Armstrong D, Galmiche JP, et al Endoscopic assessment of oesophagitis: clinical and functional correlates and further validation of the Los Angeles classification. Gut 1999;45:172-180.

  4. McKittrick JE, Lewis MW, Doane WA, Gerwig WH. The Peutz-Jeghers syndrome. Report of two cases. Arch Surg 1971;103:57-62.

  5. Abdo JM, Pérez TE, Bernal SF, Dzib SJ. Síndrome de Peutz-Jeghers. Rev Med Hosp Gen Mex 2005;68:99-105.

  6. Iannccillo H, Varsky C, Rizzolo M. Peutz-Jeghers syndrome associated with adenocarcinoma and protein-losing enteropathy. Gastrointest Endosc 2000;52:23-25.

  7. Hizawa K, Lida M, Matsumoto T. Neoplastic transformation arising in Peutz-Jeghers polyposis. Dis Colon Rectum 1993;36:953.

  8. Watne AL. Colon polyps. J Surg Onol 1997;66:201-214.

  9. Ausabarat S, Leoyklang P, Vejchapipat P, Chongsrisawat V, Suphapeetiporn K, Shotelersuk V. Novel mutations in the STK11 gene in Thai patients with Peutz-Jeghers syndrome. World J Gastroenterol 2009;15:5364-5367.

  10. Westerman AM, Wilson HP. Peutz-Jeghers syndrome: risks of a hereditary condition. Scand J Gastroenterol Suppl 1999;230:64-70.

  11. Jeghers H, McKusick VA. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits. N Engl J Med 1949;241:1031-1036.

  12. Cervantes R, Ocampo LC, Zárate F, Mata N. Síndrome de Peutz-Jeghers. Rev Gastroenterol Mex 2003;68:266-270.

  13. Wenzl JE, Bartholomew LG, Hallenbeck GA, Stickler CG. Gastrointestinal polyposis with mucocutaneous pigmentation in children (Peutz- Jeghers syndrome). Pediatrics 1961;28:655-661.

  14. Gentile AT, Bickler SW, Harrison MW, Campbell JR. Common bile duct obstruction related to intestinal polyposis in a child with Peutz- Jeghers syndrome. J Pediatr Surg 1994;29:1584-1587.

  15. Schnedl WJ, Reisinger EC, Lipp RW, Uggowitzer M, Mischinger HJ, Fickert P, et al. Biliary obstruction due to duodenojejunal intussusception in Peutz-Jeghers syndrome. J Clin Gastroenterol 1996;23:220-223.

  16. Rautou PE, Hammel P, Couvelard A, Rivet P, Aubert A, Lévy P, et al. Suspected malignant cancer of the pancreas associated with pseudoinvasive duodenal hamartomas in a patient with Peutz-Jeghers syndrome. Gastroenterol Clin Biol 2007;31:547-551.

  17. Van Coevorden F, Mathus-Vliejen EM, Brummel-Kamp W. Combined endoscopic and surgical treatment in Peutz-Jeghers syndrome. J Surg Gynecol Obstet 1986;162:426.




2020     |     www.medigraphic.com